Estenosis traqueal adquirida: estrategia diagnóstica y terapéutica / Acquired tracheal stenosis: diagnosis and treatment

Introducción. La estenosis traqueal adquirida (EA) es muy poco frecuente en la edad pediátrica pudiendo responder a múltiples causas. Mostramos nuestra experiencia en el manejo de esta patología, analizando los resultados obtenidos y estableciendo una adecuada estrategia diagnóstica y terapéutica. Material y métodos. Estudio retrospectivo de pacientes con diagnóstico broncoscópico de estenosis traqueal de causa adquirida en el período 1991-2005. Se han valorado los siguientes parámetros: sexo, edad, etiología, localización, tipo morfológico, afectación clínica, anomalías asociadas, forma de tratamiento, complicaciones, resultado y tiempo de evolución. Resultados. Diez pacientes han sido diagnosticados de EA: 5 niñas y 5 niños. La mediana de edad en el diagnóstico ha sido 6 años (rango: 1 mes-14 años). Cinco estenosis han sido postintubación, dos posquirúrgicas, una secundaria a enfermedad de Wegener, otra a cuerpo extraño enclavado en la mucosa traqueal, y el restante ha sido de causa idiopática. La localización de la lesión ha sido en tráquea cervical en 6 casos, en tercio medio en 2 y en otros dos casos traqueal distal. El tipo morfológico ha sido inflamatorio en 6 pacientes (60%), estenosis tipo membrana en dos casos y en “cuello de botella” en los dos restantes. Dos pacientes han presentado sintomatología respiratoria grave (20%) y ocho clínica moderada. Tres casos (30%) han presentado anomalías congénitas asociadas. En 9 pacientes (90%) se ha realizado un tratamiento endoscópico (dilatación, láser o prótesis) y en 4 casos (40%) cirugía (resección de la estenosis). En 3 pacientes se emplearon los dos tipos de tratamiento. El resultado ha sido satisfactorio en 9 casos (90%) y malo en uno. El tiempo de seguimiento medio ha sido de 5,3 años (rango 4 meses-10 años). Conclusiones. Aún siendo una patología muy poco frecuente, se debe descartar una estenosis traqueal en aquel paciente que presente estridor o disnea y haya sido intubado recientemente, siendo la traqueobroncoscopia el método diagnóstico de elección. La etiología de la lesión y sus características morfológicas condicionan la elección del tipo de tratamiento (AU)

Background. Acquired tracheal stenosis (ATS) is a very unfrequent lesion in the pediatric age group and may be due to diverse causes. Herein, we show our experience in the management of this entity. Patients and methods. We have reviewed the clinical charts of patients diagnosed of ATS in a single institution during the period 1991-2005. The following features have been taken into account: sex, age at diagnosis, ethiology, localization, morphologic type, symptomatology, associated anomalies, type of treatment, complications, results, and time of follow-up. Results. Ten patients were included in the study, 5 boys and 5 girls. Median age at diagnosis was 6 years (range 1month-14 years). In five cases, stenosis was due to tracheal intubation, two were postsurgical, one associated to Wegener disease, another caused by a foreign body, and the last was idyopathic. In 6 cases the lesion was located in the cervical trachea, in another two it was originated in the mid-trachea, and in the other 2 in the distal trachea. According to the morphologic type of stenosis, 6 cases were of the inflammatory type, 2 cases were of the diaphragm pattern, and another two were of the “bottle neck” type. Two patients have shown severe respiratory distress and the other 8 showed moderate symptoms. Three cases (30%) had congenital associated anomalies. In 9 patients an endoscopic treatment has been performed and 4 cases were surgically treated. In 3 cases both types of treatment were performed. Results have been satisfactory in 9 cases (90%) and bad in one patient. Mean time of follow-up has been 5,3 years (range 1 year- 10 years). Conclusions. Although it is a rare lesion, tracheal stenosis should be ruled out in every patient who has been intubated recently and shows stridor or dyspnea. Type of treatment depends on the ethiology and the morphologic characteristics of the stenosis (AU)

Tratamiento de la traqueobroncomalacia grave con prótesis traqueobronquiales endoluminales / Severe tracheobronchomalacia in children: treatment with metallic expandable stents

La traqueomalacia es una entidad poco frecuente que se caracteriza por un colapso antero-posterior de la luz traqueal durante la espiración. Se presenta generalmente asociada a atresia de esófago pero también se han descrito casos de traqueomalacia primaria y secundaria a compresiones extrínsecas. En la mayoría de las ocasiones se produce una resolución espontánea del cuadro clínico y sólo un reducido grupo de pacientes precisa tratamiento quirúrgico. Cuando la corrección quirúrgica fracasa, o está contraindicada, existe la posibilidad de colocar prótesis traqueobronquiales endoluminales (PTBE), que proporcionan un soporte interno a la vía aérea. Hemos tratado dos pacientes con cuadro PTBE metálicas expandibles: uno con traqueomalacia asociada a atresia de esófago y otro con traquebroncomalacia secundaria a compresión extrínseca por cardiomegalia y ventilación mecánica prolongada. El resultado ha sido satisfactorio en los dos casos (AU)

[Tracheal stenosis: individualized treatment]. / Estenosis traqueales: tratamiento individualizado.

INTRODUCTION: Stenosing airway disease is unfrequent in the pediatric age group and includes intraluminal obstructions, extrinsic compressions and malacias. OBJECTIVE: To show our experience in the treatment of congenital and acquired tracheal stenosis (CTS and ATS) and suprastomal tracheal collapse (STC). PATIENTS AND METHODS: Since 1990, 24 patients have been evaluated in our Unit: Ten CTS, 5 ATS and 9 STC. We have studied the following parameters: Sex, age at diagnosis, ethiology, type of lesion, associated anomalies, treatment, postoperative intubation, length of hospital stay, number of bronchoscopies, complications and follow-up. RESULTS: 14 girls and 10 boys are included in this survey. Age at diagnosis ranged from 3 days to 12 years and associated anomalies were present in 75% of cases. We have treated 22 of the 24 patients with the following procedures: Costal cartilage tracheoplasty (6 cases), slide tracheoplasty (2), resection and anastomosis (3), anterior cricoid suspension (8), dilation (2) and laser resection (1). We have achieved good results in 17 patients (77%), bad in 4 (18%) and fair in one. CONCLUSIONS: Due to the variety of stenotic lesions, we think that treatment should be tailored to each particular case and performed by a multidisciplinary pediatric team in order to achieve the best results.

Estenosis traqueales: tratamiento individualizado / Management of tracheal stenosis in children

Introducción. La patología estenosante traqueal es poco frecuente en la edad pediátrica y comprende un grupo variado de entidades que van desde las obstrucciones intraluminales hasta las compresiones extrínsecas y las malacias. Objetivo. Mostrar nuestra experiencia en el tratamiento de las estenosis traqueales congénitas (ETC) y adquiridas (EA) (obstrucciones intraluminales) y de las malacias traqueales supraestomales (MTS). Pacientes y métodos. Desde 1990, un total de 24 pacientes con patología estenosante traqueal han sido estudiados en nuestra Unidad: Diez casos de ETC, 5 de EA y 9 de MTS. En todos ellos hemos analizado los siguientes parámetros: Sexo, edad al diagnóstico, etiología, tipo de lesión, anomalías asociadas, tratamiento empleado, intubación postoperatoria, duración del ingreso, n° de broncoscopias postoperatorias; complicaciones y tiempo de seguimiento. Resultados. 14 niñas y 10 varones se incluyen en este estudio. El rango de edad en el momento del diagnóstico fue de 3 días a 12 años y el 75 por ciento de los casos han presentado anomalías asociadas. Hemos tratado 22, de los 24 pacientes, con los siguientes procedimientos: Traqueoplastia con injerto de cartílago costal (6 casos), traqueoplastia deslizada (2), resección y anastomosis T-T (3), pexia cricotraqueal (8), dilatación (2) y resección con láser (1). De forma global los resultados han sido buenos en 17 pacientes (77 por ciento), malos en 4 casos (18 por ciento) y regular en uno. Conclusiones. Dada la variedad de lesiones estenosantes y de alternativas terapéuticas existentes, creemos que para conseguir los mejores resultados, cada caso se debe abordar de forma individualizada y por un equipo pediátrico multidisciplinario (AU)

[Treatment of tracheobronchomalacia with expandable metallic stents]. / Tratamiento de la traqueobroncomalacia grave con prótesis traqueobronquiales endoluminales.

Tracheomalacia is an unfrequent disease that causes tracheal collapse during breathing. It is generally associated to esophageal atresia, but cases of primary tracheomalacia and others secondary to extrinsic compression, have also been described. Spontaneous resolution is generally the rule and only a few cases need surgical treatment. When this therapy fails or is not indicated for any reason, endoluminal tracheobronchial stents may be used. We have treated two patients with four expandable metallic stents: one had severe tracheomalacia associated to esophageal atresia and the other tracheobronchomalacia secondary to cardiomegaly. Results have been good in both cases.

Tratamiento del colapso traqueal supraestomal: alternativas quirúrgicas / Treatment of suprastomal tracheal collapse: surgical options

Fundamento. El colapso traqueal supraestomal es una de las causas de fracaso en la decanulación de pacientes traqueostomizados. Objetivo. Valorar las posibles causas y los resultados obtenidos en el tratamiento de esta complicación utilizando dos técnicas quirúrgicas diferentes: la suspensión cricotraqueal anterior y la plastia con injerto de cartílago costal.Material y métodos. Desde 1990, han presentado colapso traqueal supraestomal 9 pacientes. Hemos estudiado diversas variables incluyendo: sexo, edad, anomalías asociadas, causa de la traqueostomía, técnica quirúrgica, tiempo hasta decanulación quirúrgica, hallazgos endoscópicos, complicaciones, tiempo de evolución y resultados. Resultados. Seis niñas y 3 niños han sido estudiados por presentar colapso supraestomal grave. La edad media a la que fueron traqueotomizados era de 17 meses. La técnica quirúrgica consistió en una ventana traqueal con colgajos laterales en 6 pacientes y es desconocida en los otros 3. La indicación de la traqueostomía fue por: soporte ventilatorio prolongado (5 casos), dificultad respiratoria grave (2 casos) y estenosis subglótica (2 casos). El tiempo medio transcurrido entre la realización de la traqueostomía y la decanulación quirúrgica fue de 23 meses. En la endoscopia preoperatoria se observó en todos ellos un colapso traqueal anterior supraestomal que ocluía la luz traqueal en aproximadamente el 75 por ciento. Se ha realizado una pexia cricotraqueal anterior en 8 casos y una reconstrucción con injerto de cartílago costal en un caso. El resultado de la cirugía ha sido satisfactorio en todos los casos, si bien un paciente requirió la repetición del procedimiento de pexia, debido a colapso residual. Después de un tiempo de seguimiento medio de 45,3 meses (intervalo 1-5, 6 años), todos los pacientes se encuentran decanulados y asintomáticos. Conclusiones. Las dos técnicas correctoras ofrecen buenos resultados, pero la pexia cricotraqueal por su simplicidad es la más recomendable. En el contexto de una laringotraqueoplastia con cartílago costal en un paciente con estenosis subglótica, la estabilización de la zona malácica traqueal con el mismo cartílago es una buena alternativa (AU)

[Complete congenital cartilaginous trachea in a girl with Crouzon's syndrome]. / Tráquea cartilaginosa completa congénita en una niña con síndrome de Crouzon.

A tracheostomy was performed in a 4-month-old girl with Crouzon's syndrome because of upper respiratory obstruction. During the procedure the absence of tracheal rings was observed. These findings were confirmed by postoperative bronchoscopy. Subsequent surgical correction of the patient's craniofacial anomalies enabled decannulation when the patient was 10 months old. Complete cartilaginous trachea is very rare and is always associated with craniosynostotic syndromes. Tracheobronchial anomalies should be investigated in patients whose respiratory symptoms are not due to upper airway obstruction.

[Treatment of congenital tracheal stenosis using a slide tracheoplasty]. / Tratamiento de una estenosis traqueal congénita mediante una traqueoplastia deslizada.

Congenital tracheal stenosis (CTS) is a rare malformation that usually require corrective surgery. Resection and tracheal anastomosis is the treatment of choice when the stenosis is short. However, such an approach is not usually viable when the CTS is long, given that anastomotic pressure would be too great. New surgical techniques have allowed this serious airway malformation to be corrected satisfactorily. We report the case of a three-year-old boy with a long CTS (51% of the length of the trachea) treated with a new reconstructive technique called slide tracheoplasty. Outcome was excellent.

[Neuroblastoma IV-S. A multicenter study. Work Group of Pediatric Oncology. Spanish Society of Pediatric Surgery]. / Neuroblastoma IV-S. Estudio multicéntrico." Grupo de Trabajo de Oncología Pediátrica". Sociedad Española de Cirugía Pediátrica.

A retrospective multicentric study of 23 patients with stage IV-S neuroblastoma treated in 10 national Pediatric Surgery Departments between January 1988 and December 1992, is presented. All cases were classified as stage IV-S following staging criteria proposed by Evans et al. The age diagnosis was an average of 3 months. An acute clinical course was seen in 7 patients. Primary sites of the tumor were: adrenal gland in 6 patients (30.5%), abdominal paravertebral in 3 (13%) and unknown in 2 (9%). Distant tumor sites were: liver in 20 patients, bone marrow in 9, and skin in 5. There were not systematic therapeutic approach, in 16 cases (69%) the primary tumor was resected, 18 (78%) received chemotherapy, the liver was irradiated in 3 (13%), and 1 case (4%) received no treatment at all. One children died. The overall disease-free survival is 95.6% at 43 months post-diagnosis. A common treatment protocol is proposed, with systematic determination of N-myc oncogene which will indicate the appropriate therapy.

[Cysts of the thyroglossal duct: analysis of diagnostic errors and causes of recurrence]. / Quistes del conducto tirogloso: análisis de los errores en el diagnóstico clínico y de las causas de aparición de recurrencias.

Thyroglossal-duct cysts usually present as an anterior midline neck mass, ranking first in this location in the pediatric age. We have carried out a retrospective revision of 52 pediatric patients with a preoperative diagnosis of thyroglossal-duct cyst. All of them were treated in our surgical unit between 1982 and 1989. The male to female ratio of cases was 34:18 with symptoms appearing at an average age of 4 years. The most frequent clinical presentation was that of a cystic mass without any inflammatory signs (65%), located in the mid-line at the hyoid level (75%). In this paper we analyze the existing possibilities as regards to the clinical presentation, location and characteristics of this type of cervical swellings, as well as the most frequent causes of misdiagnosis. We also make a comparison between the results we have obtained with the different surgical techniques used. We conclude that it is necessary to perform a radical surgical treatment of the thyroglossal-duct remnants, which includes removal of the middle third of the hyoid bone and excision of the thyroglossal duct as far as the foramen cecum.

[Surgical treatment of myasthenia gravis in children using transsternal thymectomy]. / Tratamiento quirúrgico de la miastenia gravis en niños mediante timectomía transesternal.

Myasthenia gravis is an inmunological disease infrecuent during childhood. In recent years, thymectomy has gained increasing acceptance as the most effective treatment for acheiving sustained improvement in patients affected of myasthenia gravis. We report our experience in five children treated by trassternal radical thymectomy in the last five years. This patients were clinically staged following Osserman's classification as stage IIB (three cases) and stage III (two cases). All of them are in complete remission after a mean postoperative period of thirty three months. We add a review of the literature comparing the differents surgical approaches for thymectomy and its results in children.

[Conservative treatment of neonatal ovarian cysts]. / Tratamiento conservador de los quistes ováricos neonatales.

Treatment of choice of ovarian cysts diagnosed in the neonatal period has been until nowadays mainly surgical. Another possibility is a conservative approach, since these cysts have a natural tendency to spontaneous regression. We present 9 cases treated in our hospital in the last 8 years. 6 of them were treated surgically, while in 3 a conservative attitude was used, with sera ultrasound follow-up and disappearance of the cysts in 2 months in 2 cases and in 12 in the other. In cysts smaller than 5 cm we adopt a conservative management, leaving surgery only for those bigger or complicated.

[Congenital cystic adenomatoid malformation of the lung (author's transl)]. / Adenomatosis quística pulmonar congénita. Estudio de cuatro casos.

Four cases of congenital cystic adenomatoid malformation of the lung are described. One was a stillborn, and the other three newborns, all of them surviving after lobectomy. Pathological, clinical and radiological features are discussed and compared with previously reported cases.